POS1448 INDIVIDUALS AT RISK FOR SJÖGREN’S SYNDROME – TWO-YEAR DATA OF THE PRESSTIGE (THE PRE-SJÖGREN SYNDROME TARGETED IMMUNOLOGY EVALUATION) STUDY
Authors: S. Zenz, P. Javorova, L. Erlacher, B. Dreo, A. Lackner, D. Cornec, J. Thiel, M. Stradner
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease often diagnosed years after appearance of the first symptoms. Therefore, little is known about initial development of the disease. Anti-Ro antibodies can be found years before the first symptoms appear. <h3>Objectives</h3> To identify individuals at risk for pSS and follow up over 10 years to examine symptoms and immunopathology before and during the development of pSS. <h3>Methods</h3> This ongoing longitudinal study enrolled individuals at risk for developing pSS but have not met the ACR-EULAR classification criteria. We recruited three groups: A.) Anti-Ro positive individuals without any symptoms for an underlying systemic autoimmune disease; B.) First degree relatives of patients with a diagnosis of pSS AND an abnormal immunologic laboratory value (ANA ≥ 1:160 and/or anti-Ro<sup>+</sup> and/or rheumatoid factor<sup>+</sup>) C.) Individuals with at least one feature of the ACR-EULAR classification criteria for pSS, but not fulfilling the criteria. At screening and at annual follow-up visits, demographic data, blood, urine and saliva samples were obtained. Salivary flow, Schirmer's test, and salivary gland ultrasonography (SGUS) using Hocevar-Score were performed. A lip salivary gland biopsy (LSB) was obtained at the screening visit and repeated once when typical symptoms appeared within the follow-up visits. The primary endpoint was the development of definite pSS according to the ACR-EULAR classification criteria or a diagnosis of another systemic autoimmune disease. <h3>Results</h3> After two years, 53 subjects were recruited from all three groups, of whom 31 were enrolled in the study (Anti-SSA<sup>+</sup> n=25, relatives n=4, incomplete n=2). Most of the 22 screening failures were relatives who did not have an abnormal immunologic laboratory value. 28 individuals were female, they had a mean age of 49.6 ± 12.7 years (SD; standard deviation) and a mean body weight of 71.4 ± 15 kg. 11 individuals reported having a relative with an underlying autoimmune disease. The median ESSDAI was 0 (0-5; min-max). individuals had a pathological stimulated whole saliva flow (mean 3.7 ± 1.4 g) and eight had pathological Schirmer's test (left eye: mean 13.6 ± 13.1 mm; right eye: 13.2 ±12.2 mm). Three of 14 individuals had a pathological SGUS score. All three individuals were recruited in the Anti-Ro<sup>+</sup> group. At screening visit, 11 LSB were performed (mean focus score of 0.53 ± 1.2) and one individual reached a focus score ≥ 1. So far, 18 individuals have completed the two-year visit. Two of them had pathological stimulated whole saliva flow (mean 3.85 ± 1.4 g) and five of them a pathological Schirmer's test (left eye: mean 15.4 ± 13 mm; right eye: 14.5 ± 13). The median ESSDAI was 0 [0-4]. Eight SGUS were performed with a mean Hocevar-Score of 5.6 ± 8. Within two years, four patients (13%) were diagnosed with pSS, one (3%) with systemic lupus erythematosus and one (3%) with rheumatoid arthritis. <h3>Conclusion</h3> This longitudinal study for the first time provides us with data on the evolution of pSS. Future recruitment at multiple centers and longer follow-ups will shed light on the earliest phase of pSS. <h3>REFERENCES:</h3> NIL. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.